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Oligomerization propensity and flexibility of yeast frataxin studied by X-ray crystallography and small-angle X-ray scattering.

Söderberg CA, Shkumatov AV, Rajan S, Gakh O, Svergun DI, Isaya G, Al-Karadaghi S
J Mol Biol 414 783-97 (2011)
Cited: 22 times
EuropePMC logo PMID: 22051511

Abstract

Frataxin is a mitochondrial protein with a central role in iron homeostasis. Defects in frataxin function lead to Friedreich's ataxia, a progressive neurodegenerative disease with childhood onset. The function of frataxin has been shown to be closely associated with its ability to form oligomeric species; however, the factors controlling oligomerization and the types of oligomers present in solution are a matter of debate. Using small-angle X-ray scattering, we found that Co(2+), glycerol, and a single amino acid substitution at the N-terminus, Y73A, facilitate oligomerization of yeast frataxin, resulting in a dynamic equilibrium between monomers, dimers, trimers, hexamers, and higher-order oligomers. Using X-ray crystallography, we found that Co(2+) binds inside the channel at the 3-fold axis of the trimer, which suggests that the metal has an oligomer-stabilizing role. The results reveal the types of oligomers present in solution and support our earlier suggestions that the trimer is the main building block of yeast frataxin oligomers. They also indicate that different mechanisms may control oligomer stability and oligomerization in vivo.

Reviews - 3oeq mentioned but not cited (2)

  1. Neurodegeneration in Friedreich's ataxia: from defective frataxin to oxidative stress. Gomes CM, Santos R. Oxid Med Cell Longev 2013 487534 (2013)
  2. Tangled web of interactions among proteins involved in iron-sulfur cluster assembly as unraveled by NMR, SAXS, chemical crosslinking, and functional studies. Kim JH, Bothe JR, Alderson TR, Markley JL. Biochim Biophys Acta 1853 1416-1428 (2015)

Articles - 3oeq mentioned but not cited (10)

  1. The Structure of the Complex between Yeast Frataxin and Ferrochelatase: CHARACTERIZATION AND PRE-STEADY STATE REACTION OF FERROUS IRON DELIVERY AND HEME SYNTHESIS. Söderberg C, Gillam ME, Ahlgren EC, Hunter GA, Gakh O, Isaya G, Ferreira GC, Al-Karadaghi S. J Biol Chem 291 11887-11898 (2016)
  2. Missense mutations linked to friedreich ataxia have different but synergistic effects on mitochondrial frataxin isoforms. Li H, Gakh O, Smith DY, Ranatunga WK, Isaya G. J Biol Chem 288 4116-4127 (2013)
  3. Oligomerization propensity and flexibility of yeast frataxin studied by X-ray crystallography and small-angle X-ray scattering. Söderberg CA, Shkumatov AV, Rajan S, Gakh O, Svergun DI, Isaya G, Al-Karadaghi S. J Mol Biol 414 783-797 (2011)
  4. Architecture of the Human Mitochondrial Iron-Sulfur Cluster Assembly Machinery. Gakh O, Ranatunga W, Smith DY, Ahlgren EC, Al-Karadaghi S, Thompson JR, Isaya G. J Biol Chem 291 21296-21321 (2016)
  5. The molecular basis of iron-induced oligomerization of frataxin and the role of the ferroxidation reaction in oligomerization. Söderberg CAG, Rajan S, Shkumatov AV, Gakh O, Schaefer S, Ahlgren EC, Svergun DI, Isaya G, Al-Karadaghi S. J Biol Chem 288 8156-8167 (2013)
  6. Architecture of the Yeast Mitochondrial Iron-Sulfur Cluster Assembly Machinery: THE SUB-COMPLEX FORMED BY THE IRON DONOR, Yfh1 PROTEIN, AND THE SCAFFOLD, Isu1 PROTEIN. Ranatunga W, Gakh O, Galeano BK, Smith DY, Söderberg CA, Al-Karadaghi S, Thompson JR, Isaya G. J Biol Chem 291 10378-10398 (2016)
  7. Zinc and the iron donor frataxin regulate oligomerization of the scaffold protein to form new Fe-S cluster assembly centers. Galeano BK, Ranatunga W, Gakh O, Smith DY, Thompson JR, Isaya G. Metallomics 9 773-801 (2017)
  8. Thermometer: a webserver to predict protein thermal stability. Miotto M, Armaos A, Di Rienzo L, Ruocco G, Milanetti E, Tartaglia GG. Bioinformatics 38 2060-2061 (2022)
  9. Heat and cold denaturation of yeast frataxin: The effect of pressure. Puglisi R, Cioni P, Gabellieri E, Presciuttini G, Pastore A, Temussi PA. Biophys J 121 1502-1511 (2022)
  10. SAXS and stability studies of iron-induced oligomers of bacterial frataxin CyaY. Fekry M, Alshokry W, Grela P, Tchórzewski M, Ahlgren EC, Söderberg CA, Gakh O, Isaya G, Al-Karadaghi S. PLoS One 12 e0184961 (2017)


Reviews citing this publication (3)

  1. Frataxin: a protein in search for a function. Pastore A, Puccio H. J Neurochem 126 Suppl 1 43-52 (2013)
  2. Iron-sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia. Vaubel RA, Isaya G. Mol Cell Neurosci 55 50-61 (2013)
  3. Structural aspects of enzymes involved in prokaryotic Gram-positive heme biosynthesis. Falb N, Patil G, Furtmüller PG, Gabler T, Hofbauer S. Comput Struct Biotechnol J 21 3933-3945 (2023)

Articles citing this publication (7)

  1. Structure of Actin-related protein 8 and its contribution to nucleosome binding. Gerhold CB, Winkler DD, Lakomek K, Seifert FU, Fenn S, Kessler B, Witte G, Luger K, Hopfner KP. Nucleic Acids Res 40 11036-11046 (2012)
  2. Architectural Features of Human Mitochondrial Cysteine Desulfurase Complexes from Crosslinking Mass Spectrometry and Small-Angle X-Ray Scattering. Cai K, Frederick RO, Dashti H, Markley JL. Structure 26 1127-1136.e4 (2018)
  3. Iron-induced oligomerization of human FXN81-210 and bacterial CyaY frataxin and the effect of iron chelators. Ahlgren EC, Fekry M, Wiemann M, Söderberg CA, Bernfur K, Gakh O, Rasmussen M, Højrup P, Emanuelsson C, Isaya G, Al-Karadaghi S. PLoS One 12 e0188937 (2017)
  4. Structural characterization of metal binding to a cold-adapted frataxin. Noguera ME, Roman EA, Rigal JB, Cousido-Siah A, Mitschler A, Podjarny A, Santos J. J Biol Inorg Chem 20 653-664 (2015)
  5. The role of the N-terminal tail for the oligomerization, folding and stability of human frataxin. Faraj SE, Venturutti L, Roman EA, Marino-Buslje CB, Mignone A, Tosatto SC, Delfino JM, Santos J. FEBS Open Bio 3 310-320 (2013)
  6. A nucleotide-dependent oligomerization of the Escherichia coli replication initiator DnaA requires residue His136 for remodeling of the chromosomal origin. Saxena R, Stanley CB, Kumar P, Kumar P, Cuneo MJ, Patil D, Jha J, Weiss KL, Chattoraj DK, Crooke E. Nucleic Acids Res 48 200-211 (2020)
  7. Metal Ion Binding in Wild-Type and Mutated Frataxin: A Stability Study. Morante S, Botticelli S, Chiaraluce R, Consalvi V, La Penna G, Novak L, Pasquo A, Petrosino M, Proux O, Rossi G, Salina G, Stellato F. Front Mol Biosci 9 878017 (2022)


Related citations provided by authors (1)

  1. The structures of frataxin oligomers reveal the mechanism for the delivery and detoxification of iron. Karlberg T, Schagerlof U, Gakh O, Park S, Ryde U, Lindahl M, Lleath K, Garman E, Isaya G, Al-Karadaghi S Structure 14 1535- (2006)

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